Sprengel deformity is usually noticed at birth and has both cosmetic and functional implications. The elevated scapula is visually noticeable and there is an associated restriction in the motion of the scapula and glenohumeral joint.
The Cavendish classification 2,6 is one method used for grading:
very mild deformity is observed
when covered with clothes the deformity is almost invisible
the deformity is still mild but appears as a bump
the superomedial portion of the high scapula is convex, forming a bump
moderate deformity with 2-5 cm of visible elevation of the affected shoulder
severe deformity with >5 cm elevation of the affected shoulder, accompanied by neck webbing
The abnormality results from failure of caudal migration of the scapula during early fetal development.
Sprengel deformities usually coexist with other congenital abnormalities, particularly those involving the vertebrae and ribs. An omovertebral bar (fibrous, cartilaginous and/or osseous connection between the scapula and cervical spine) is often present.
It is also commonly associated with hypoplasia or atrophy of regional muscles, and these associated features can cause further misshaping of the shoulder and limitation of shoulder movement.
Patients with Sprengel deformity often have one or more of the following abnormalities and conditions:
underdevelopment of clavicle or humerus
These possible co-existing anomalies need to be looked for in any patient presenting with Sprengel deformity.
The affected scapula is elevated and rotated, with the inferior angle directed laterally.
The radiographic Rigault classification
grade I: superomedial angle lower than T2 but above T4 transverse process
grade II: superomedial angle located between C5 and T2 transverse process
grade III: superomedial angle above C5 transverse process
CT with 3D reconstruction is being used to evaluate omovertebral connection and scapula dysplasia and malpositioning. It can be used in preoperative planning.
There may be a role in MRI to assess omovertebral connection
Treatment and prognosis
Surgery is performed to improve cosmetic and functional disability. It is generally considered for patients between 3 and 8 years of age who have moderate to severe disability (or a Cavendish score of 3-4) 1.
Two of the most used surgical methods are the “Woodward” procedure and the “modified-Green” procedure with good functional and cosmetic outcome.
History and etymology
It is named after Otto Gerhard Karl Sprengel (1852-1915), a German surgeon who described four cases in 1891.